APERTS-SYNDROME - CEPHALOMETRIC EVALUATION AND CONSIDERATIONS ON PATHOGENESIS

Apert's syndrome is a malformation characterized by abnormalities in t he cranial vault, midfacial malformations, and syndactylia. The presen t study analyzes the lateral and frontal projections of the tele-radio grams from five patients. Data were taken on the skeletal features and an attempt was made to interpret them in terms of functional matrices . We have used cephalometry as a descriptive device although the inten t was also to use it to provide a perspective on the pathogenetic data derived from the literature. The hypothesis analyzed is that from the outset of craniofacial pathogenesis there may be a primitive alterati on of the cartilaginous template from which are derived endochondral b ones. The progressive involvement of the synchondrosis of the cranial base is subsequently transmitted to the membranous structures of the v ault and face through the coronal ring and lambdoid suture systems. Al though the data gained do not actually confirm this hypothesis, they d o provide further support for it.